Fli1 deficiency and beyond: a unique pathway linking peripheral vasculopathy and dermal fibrosis in systemic sclerosis

Systemic sclerosis (SSc), or scleroderma, is a multisystem autoimmune connective tissue disease of unknown cause characterized by early immune activation and microvascular injury followed by fibrosis of the skin and multiple internal organs (1,2). In the course of SSc, progressive fibrosis disrupts the architecture of the affected tissues and ultimately leads to organ dysfunction and substantial mortality (1). The concomitant obliterative vasculopathy, characterized by subendothelial intimal fibrosis and proliferation, and the progressive loss of peripheral microvessels and lack of compensatory angiogenesis may lead to chronic tissue ischemia with severe vascular complications, such as digital ulcers and gangrene (2). This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.